Myelofibrosis laboratory tests

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Peripheral blood smear and bone marrow examination helps in making the diagnosis of myelofibrosis. Various tests performed to aid in reaching the diagnosis include complete blood count, peripheral blood smear and bone marrrow examination, complete metabolic panel, and leukocyte alkaline phosphatase test.

Laboratory Findings

Complete Blood Count

Peripheral Blood Smear

Bone Marrow Biopsy

  • Hypercellularity and increased numbers of megakaryocytes[18]
  • Increased microvascular density, bizarre vessel architecture, and increased number of pericytes[19][20]
  • Panhyperplasia, increase in the reticular fibers, myeloid fibrosis, and osteosclerosis[21]
  • Fibroblast-like and myofibroblast-like reticulum cells[22]
  • Proplatelet (pseudopodia of megakaryocyte which extend into bone marrow sinuses to release platelets) formation[17]
  • Vessels show increased density, tortuous architecture, and increased branching[20]
  • Lymphoid nodules

Comprehensive Metabolic Panel

  • Elevated bilirubin[23]
  • Elevated alkaline phosphatase (ALP)

Leukocyte Alkaline Phosphatase Test

Other Findings

  • Elevated serum lactate dehydrogenase[25][28]
  • Elevated lactic acid level[29]
  • Elevated uric acid
  • Elevated serum glutamic oxaloacetic transaminase level
  • Decreased total cholesterol
  • Diminished high to low-density lipoprotein cholesterol ratio
  • Prolongation of prothrombin time (PT)

References

  1. Thiele J, Kvasnicka HM (August 2005). "Hematopathologic findings in chronic idiopathic myelofibrosis". Semin. Oncol. 32 (4): 380–94. doi:10.1053/j.seminoncol.2005.04.010. PMID 16202684.
  2. Cervantes F, Correa JG, Hernandez-Boluda JC (May 2016). "Alleviating anemia and thrombocytopenia in myelofibrosis patients". Expert Rev Hematol. 9 (5): 489–96. doi:10.1586/17474086.2016.1154452. PMID 26891375.
  3. Hernández-Boluda JC, Correa JG, García-Delgado R, Martínez-López J, Alvarez-Larrán A, Fox ML, García-Gutiérrez V, Pérez-Encinas M, Ferrer-Marín F, Mata-Vázquez MI, Raya JM, Estrada N, García S, Kerguelen A, Durán MA, Albors M, Cervantes F (April 2017). "Predictive factors for anemia response to erythropoiesis-stimulating agents in myelofibrosis". Eur. J. Haematol. 98 (4): 407–414. doi:10.1111/ejh.12846. PMID 28009442.
  4. Barraco D, Lasho TL, Gangat N, Finke C, Elala YC, Pardanani A, Tefferi A (June 2016). "Leukocytosis and presence of CALR mutation is associated with non-hepatosplenic extramedullary hematopoiesis in primary myelofibrosis". Blood Cancer J. 6: e436. doi:10.1038/bcj.2016.44. PMC 5141359. PMID 27315113.
  5. Beauverd Y, Alimam S, McLornan DP, Radia DH, Harrison CN (October 2016). "Disease characteristics and outcomes in younger adults with primary and secondary myelofibrosis". Br. J. Haematol. 175 (1): 37–42. doi:10.1111/bjh.14173. PMID 27293069.
  6. Ota S, Hiramatsu Y, Kondo E, Kasahara A, Takada S, Umena S, Noguchi T, Tanimoto M, Matsumura T (December 2014). "Severe case of peripheral leukocytosis initially diagnosed as myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable, but possibly prefibrotic primary myelofibrosis". Acta Med. Okayama. 68 (6): 363–8. doi:10.18926/AMO/53025. PMID 25519030.
  7. 7.0 7.1 Scotch AH, Kosiorek H, Scherber R, Dueck AC, Slot S, Zweegman S, Boekhorst P, Commandeur S, Schouten H, Sackmann F, Fuentes AK, Hernández-Maraver D, Pahl HL, Griesshammer M, Stegelmann F, Döhner K, Lehmann T, Bonatz K, Reiter A, Boyer F, Etienne G, Ianotto JC, Ranta D, Roy L, Cahn JY, Harrison CN, Radia D, Muxi P, Maldonado N, Besses C, Cervantes F, Johansson PL, Barbui T, Barosi G, Vannucchi AM, Paoli C, Passamonti F, Andreasson B, Ferrari ML, Rambaldi A, Samuelsson J, Birgegard G, Xiao Z, Xu Z, Zhang Y, Sun X, Xu J, Kiladjian JJ, Zhang P, Gale RP, Mesa RA, Geyer HL (December 2017). "Symptom burden profile in myelofibrosis patients with thrombocytopenia: Lessons and unmet needs". Leuk. Res. 63: 34–40. doi:10.1016/j.leukres.2017.10.002. PMID 29096334. Vancouver style error: initials (help)
  8. Wassie E, Finke C, Gangat N, Lasho TL, Pardanani A, Hanson CA, Ketterling RP, Tefferi A (April 2015). "A compendium of cytogenetic abnormalities in myelofibrosis: molecular and phenotypic correlates in 826 patients". Br. J. Haematol. 169 (1): 71–6. doi:10.1111/bjh.13260. PMID 25521305.
  9. Guglielmelli P, Rotunno G, Pacilli A, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pieri L, Fjerza R, Pietra D, Cilloni D, Sant'Antonio E, Salmoiraghi S, Passamonti F, Rambaldi A, Barosi G, Barbui T, Cazzola M, Vannucchi AM (September 2016). "Prognostic impact of bone marrow fibrosis in primary myelofibrosis. A study of the AGIMM group on 490 patients". Am. J. Hematol. 91 (9): 918–22. doi:10.1002/ajh.24442. PMID 27264006.
  10. Xu Z (February 2017). "MDS/MPN with ring sideroblasts and thrombocytosis masquerading as prefibrotic/early primary myelofibrosis". Blood. 129 (5): 657. doi:10.1182/blood-2016-11-749937. PMID 28153839.
  11. Cheminant M, Delarue R (August 2013). "[Investigation and management of patients presenting with thrombocytosis]". Rev Med Interne (in French). 34 (8): 465–71. doi:10.1016/j.revmed.2013.02.020. PMID 23498669.
  12. Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  13. Egelé A, van Gelder W, Riedl J (December 2015). "Automated detection and classification of teardrop cells by a novel RBC module using digital imaging/microscopy". Int J Lab Hematol. 37 (6): e153–6. doi:10.1111/ijlh.12399. PMID 26118701.
  14. Tóth P, Tóth Z (2002). "Idiopathic myelofibrosis with prominent postsplenectomy erythroblastosis terminating in acute myeloid transformation". Haematologia (Budap). 32 (2): 155–61. PMID 12412736.
  15. Osman Y, Kishi K, Narita M, Saito H, Masuko M, Koike T, Shibata A (June 1996). "Idiopathic myelofibrosis with unusually high erythroblastosis in the peripheral blood". Am. J. Hematol. 52 (2): 122–3. doi:10.1002/(SICI)1096-8652(199606)52:2<122::AID-AJH12>3.0.CO;2-J. PMID 8638637.
  16. Reilly JT, Barnett D, Dolan G, Forrest P, Eastham J, Smith A (January 1993). "Characterization of an acute micromegakaryocytic leukaemia: evidence for the pathogenesis of myelofibrosis". Br. J. Haematol. 83 (1): 58–62. PMID 8435338.
  17. 17.0 17.1 Muth M, Büsche G, Bock O, Hussein K, Kreipe H (November 2010). "Aberrant proplatelet formation in chronic myeloproliferative neoplasms". Leuk. Res. 34 (11): 1424–9. doi:10.1016/j.leukres.2010.03.040. PMID 20430444.
  18. Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  19. Madelung A, Bzorek M, Bondo H, Zetterberg E, Bjerrum OW, Hasselbalch HC, Scheding S, Ralfkiaer E (March 2012). "A novel immunohistochemical sequential multi-labelling and erasing technique enables epitope characterization of bone marrow pericytes in primary myelofibrosis". Histopathology. 60 (4): 554–60. doi:10.1111/j.1365-2559.2011.04104.x. PMID 22250648.
  20. 20.0 20.1 Lundberg LG, Lerner R, Sundelin P, Rogers R, Folkman J, Palmblad J (July 2000). "Bone marrow in polycythemia vera, chronic myelocytic leukemia, and myelofibrosis has an increased vascularity". Am. J. Pathol. 157 (1): 15–9. doi:10.1016/S0002-9440(10)64511-7. PMC 1850191. PMID 10880370.
  21. Milanesi B, Pirali F (September 1981). "[Laboratory diagnosis of idiopathic myelofibrosis. Review of the literature and presentation of 15 cases]". Minerva Med. (in Italian). 72 (34): 2265–72. PMID 7290453.
  22. Biagini G, Severi B, Govoni E, Preda P, Pileri S, Martinelli G, Visani G, Finelli C, Castaldini C (1985). "Stromal cells in primary myelofibrosis: ultrastructural observations". Virchows Arch., B, Cell Pathol. 48 (1): 1–8. PMID 2580387.
  23. Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  24. Michiels JJ, Bernema Z, Van Bockstaele D, De Raeve H, Schroyens W (March 2007). "Current diagnostic criteria for the chronic myeloproliferative disorders (MPD) essential thrombocythemia (ET), polycythemia vera (PV) and chronic idiopathic myelofibrosis (CIMF)". Pathol. Biol. 55 (2): 92–104. doi:10.1016/j.patbio.2006.06.002. PMID 16919893.
  25. 25.0 25.1 Thiele J, Kvasnicka HM, Zankovich R, Diehl V (May 2002). "Early-stage idiopathic (primary) myelofibrosis--current issues of diagnostic features". Leuk. Lymphoma. 43 (5): 1035–41. PMID 12148883.
  26. Chikkappa G, Pasquale DT, Wang GJ, Santella D (May 1989). "Monocyte induces alkaline phosphatase synthesis in neutrophils of chronic myeloid leukemia and myelofibrosis with myeloid metaplasia patients". Am. J. Clin. Pathol. 91 (5): 550–7. PMID 2718954.
  27. Mariańska B, Leszko B, Maj S, Apel D (1987). "[Alkaline phosphatase activity of the granulocytes in polycythemia vera and myelofibrosis]". Acta Haematol Pol (in Polish). 18 (1–2): 23–7. PMID 3434154.
  28. Kamiunten A, Shide K, Kameda T, Ito M, Sekine M, Kubuki Y, Hidaka T, Akizuki K, Tahira Y, Toyama T, Kawano N, Marutsuka K, Maeda K, Takeuchi M, Kawano H, Sato S, Ishizaki J, Shimoda H, Yamashita K, Matsuoka H, Shimoda K (July 2018). "Early/prefibrotic primary myelofibrosis in patients who were initially diagnosed with essential thrombocythemia". Int. J. Hematol. doi:10.1007/s12185-018-2495-2. PMID 29987745.
  29. Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.


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